• Amalia Colalillo, Eric Hachulla, Chiara Pellicano, Vanessa Smith, Christina Bergmann, Gabriela Riemekasten, Elisabetta Zanatta, Jörg Henes, David Launay, Antonella Marcoccia, Ana Maria Gheorghiu, Marie-Elise Truchetet, Florenzo Iannone, Carmen Pilar Simeón Aznar, Susana Oliveira, Madelon Vonk, Francesco Del Galdo, Edoardo Rosato, and EUSTAR Collaborators.
• Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.
• Chest. 2024 Oct 1; 166 (4): 837844837-844.

BackgroundThe 2022 European Society of Cardiology/European Respiratory Society guidelines define pulmonary hypertension (PH) as a resting mean pulmonary artery pressure (mPAP) > 20 mm Hg at right heart catheterization (RHC). Previously, patients with an mPAP between 21 and 24 mm Hg were classified in a "gray zone" of unclear clinical significance.Research QuestionWhat is the diagnostic performance of the main parameters used for PH screening in detecting patients with systemic sclerosis (SSc) with an mPAP of 21 to 24 mm Hg at RHC?Study Design And MethodsPatients with SSc from the European Scleroderma Trials and Research (EUSTAR) database with available tricuspid annular plane systolic excursion (TAPSE), systolic PAP (sPAP), and mPAP data were included. Patients with mPAP 21 to 24 mm Hg and patients with mPAP ≤ 20 mm Hg were considered for the analysis. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated.ResultsTAPSE/sPAP was lower in the group of patients with SSc with mPAP 21 to 24 mm Hg than in the non-PH group (0.58 [0.46-0.72] vs 0.69 [0.57-0.81] mm/mm Hg, respectively; P < .01). No difference was found in other parameters between the two groups. Diffusing capacity of the lungs for carbon monoxide < 80% of the predicted value had the highest sensitivity (88.9%) and NPV (80%), but the lowest specificity (18.2%) and PPV (30.8%) in detecting patients with SSc with mPAP 21 to 24 mm Hg. TAPSE/sPAP < 0.55 mm/mm Hg had the highest specificity (78.9%), PPV (50%), and accuracy (68.1%); its NPV was 75.4%, and its sensitivity was 45.1%.InterpretationIn this study, diffusing capacity of the lungs for carbon monoxide < 80% of the predicted value was the parameter with the highest sensitivity and NPV in detecting patients with SSc with mPAP 21 to 24 mm Hg. TAPSE/sPAP < 0.55 mm/mm Hg had the highest specificity, PPV, and accuracy and, therefore, can be a useful additional parameter to decrease the number of unnecessary RHCs.Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.

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